Best Practice Guidance for the management of hearing issues in Down syndrome

Background

Up to 50-70%of people with Down syndrome will experience impaired hearing at some point in their life1 - 4. Hearing losses may be conductive, sensorineural or mixed in nature, and may be temporary or permanent. The patterns of hearing loss presenting in Down syndrome change throughout life, with otitis media with effusion (glue ear) the most common cause in childhood, and sensorineural deafness becoming more prevalent with age.

Otitis media with effusion (OME or glue ear) affects up to 35% of children with Down syndrome at birth and 93% at 1 year old, reducing to 68% and 38% by the ages of 5 and 8 respectively 6.7,8. There is a higher incidence of ossicular abnormalities in Down syndrome which may present with a conductive hearing loss 9.The incidence of sensorineural hearing loss identified at newborn hearing screening in children with Down syndrome is higher than in the general population at 4-6% 3, 8.Presbyacusis (age-related sensorineural hearing loss) is more common, and develops significantly earlier in adults with Down syndrome 1-4

Early hearing loss has a significant impact on the speech and language development of children with Down syndrome, resulting in a disproportionately more severe speech delay than would be predicted by IQ alone 5.It is important to consider hearing loss, either as a cause or an exacerbating factor, as a part of an assessment of behavioural problems, mental health or cognitive decline in children and adults with Down syndrome.

Health care professionals need to have a heightened awareness of the possibility of hearing loss, with the aim of identifying any impairment and offer appropriate interventionearly, to maximise the educational and social development of children, and enabling independence and quality of life for adults.

It is important to take into consideration, at all ages, the person’s communication needs, any additional medical needsand developmental and behavioural phenotype when assessing and offering an intervention for the management of their hearing difficulties.

Recommendation:

A multidisciplinary team approach should be applied for the management of hearing issues in the child with Down syndrome. This should include a paediatric audiologist, otolaryngologistand paediatrician, with strong links to speech and language therapists and teachers for the deaf. Correspondence should be routinely shared with the parents/carers and, with parents’ consent, with all members of the team 6.

Hearing Screening & Surveillance:

Studies have shown a poor correlation between caretakers’ perception of hearing loss and objective hearing loss in children with Down syndrome, therefore regular objective hearing assessments should be performed as parents may underestimate hearing loss 10.

Hearing Assessment:

At all ages, people with Down syndrome have narrow ear canals which predispose to accumulation of wax. This may affect impedance testing and hearing 14.

Most people with Down syndrome are able to respond to standard test procedures e.g. distraction, speech discrimination, pure tone audiometry (play or standard) or visual reinforcement audiometrywhencarried out by testers with expertise in working with people with learning disability. However in the young infant it can be a challenge to undertake these tests. Where a dual diagnosis, e.g. autism, visual impairment etc. exists, the assessments can be particularly challenging.

Threshold measurement tests appropriate to developmental age must be used. Objective audiometry e.g. brain-stem evoked audiometry, may be required if a hearing level cannot be established reliably 11, 12, 13.

Because of an increased incidence of sensorineural as well as conductive loss, the frequency range tested should include 8000Hz whenever feasible, as this may be an early warning of impending sensorineural deafness 14, 15.

Recommendation:

All children with Down syndrome should have new-born hearing screening followed by targeted hearing assessments, initially at 6 to 10 months and then six monthly till the age of two and to continue at least annually throughout school-age years.

A hearing assessment is recommended at least two yearly throughout adult life or more frequently if there are concerns. This should include measurement of auditory thresholds, impedance testing (tympanometry) and otoscopy.

Guidance for management of hearing issues:

  1. Children and adults with Down syndrome have relatively narrow ear canals predisposing them to accumulation of wax, which may affect hearing and impedance testing. Regular dewaxing should be part of their management. This is particularly important as progressive middle ear disease or cholesteatoma can be missed, if wax is not actively managed.
  2. Otitis media with effusion (OME, glue ear) can be managed conservatively, with hearing aids (traditional hearing aids applied behind the ear or the BAHA Soft Band) or with ventilation tubes (grommets) 16 - 19. Each child’s case should be considered on its own merits by a multidisciplinary team with experience in dealing with glue ear in children with Down syndrome.
  3. NICE Guidelines (2008) recommend hearing aids as the first-line treatment for hearing loss associated with glue ear in children with Down syndrome16.
  4. Factors that should be considered when discussing the use of hearing aids versus grommets (ventilation tubes) for the management of glue ear include the severity of hearing loss, the presence of recurrent acute otitis media (where grommet insertion is beneficial), the age of the child, the practicality of grommet insertion and the risks associated with grommets e.g. infection, perforation of tympanic membraneand the views of the family.16,20.
  5. Children with Down syndrome often have narrow ear canals and smaller grommets may need to be used, with the associated disadvantage of earlier extrusion.
  6. Adenoidectomy should be considered in thesurgical management of recurrent OME, recurrent acute otitis media and also as part of the management of chronic nasal obstruction and obstructive sleep apnoea. The indications remain the same as those in a typically developing child.
  1. Significant laryngopharyngeal reflux has also been shown to predispose to OME in children, which may be exaggerated in Down syndrome secondary to hypotonia 21.
  2. At all ages, particular attention should be paid to the assessment and treatment of suppurative nasal and ear conditions, with the aim of reducing acute middle ear infections and exclusion of complications of chronic eustachian tube dysfunction e.g. cholesteatoma.
  3. The BAHA Soft Band and bone-anchored hearing aids have an important role in the management of conductive hearing loss where grommets and conventional hearing aids cannot be tolerated or have failed 22.
  4. All people with Down syndrome with sensorineural loss should be actively encouraged to use of hearing aids at all ages. Cochlear implantation has been shown to be successful in children with Down syndrome and referrals should be made based on the same audiological referral criteria as the typically developing child 17, 19, 20, 23.
  5. People with Down syndrome should have life-long audiological follow-up with rapid access to services including speech and language therapy to enable them to meet their developmental potential and avoid social exclusion 5.
  6. Transition of care from paediatric audiological services to adult services should involve direct transfer of care to a named professional

Additional useful resources for parents is provided by the National Deaf Children’s Society:

(Last accessed 31.12.2016)

References

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  2. Davies B.(1998) Auditory disorders in Down’s syndrome. Scand Audiol Suppl. 30:65-68.
  3. Park AH, Wilson MA, Stevens PT Harward R, Hohler N. (2012) Identification of hearing loss in pediatric patients with Down syndrome. Otolaryngol Head NeckSurg.146:135-140.
  4. Meuwese-Jongejeugd A, Vink M, van Zanten B, Verschuure H, Eichhorn E, Koopman D, Bernsen R, Evenhuis H.(2006)Prevalence of hearing loss in 1598 adults with an intellectual disability: cross-sectional population based study.Int J Audiol;45(11):660-9.
  5. Laws G, Hall A (2014) Early hearing loss and language abilities in children with Down syndrome. Int J Lang Commun Disord; 49(3) 333-42
  6. Fortnum H, Leighton P, Smith MD, Brown L, Jones M, Benton C, et al. Assessment of the feasibility and clinical value of further research to evaluate the management options for children with Down syndrome and otitis media with effusion: a feasibility study. Health Technol Assess 2014;18 (60).
  7. Austeng, M. E., Akre, H.,Øverland B., Abdelnoor M., Falkenberg, E-S.,Kværner K. J. (2013). Otitis media with effusion in children with in Down syndrome. Int J PedOtorhinolaryngol. 77(8):1329-32
  8. Tedeschi A S., Roizen N J., Taylor H G., Murray G., Curtis CA., Parikh AS. (2014). The prevalence of Congenital Hearing Loss in Neonates with DownSyndrome. J Pediatrics , 166(1):168 - 171.
  9. Intrapiromkul J, Aygun N, Tunkel DE, Carone M, Yousem DM.Inner ear anomalies seen on CT images in people with Down syndrome.Pediatr Radiol 2012 Dec;42(12):1449-55.
  10. Lau WL, Ko CH, Cheng WW.Prevalence and parental awareness of hearing loss in children with Down syndrome. (2015)See comment in PubMed Commons belowChin Med J (Engl). 20;128(8):1091-5
  11. Evenhuis,HM. (1996) Dutch consensus on diagnosis and treatment of hearing impairment in children and adults with intellectual disability. J Intel Disabil Res40(1) :451-456.
  12. Sonsken, PM. (1985) A developmental reappraisal of clinical tests of hearing for normal and handicapped children. Part 3.The handicapped child. Mat and Ch Health. June 1985. 170-175
  13. Widen, JE,Folsom, RC,Thompson, G, Wilson, WR.(1987) Auditory brainstem responses in young adults with Down syndrome. Am J Mental Deficiency91.472-479
  14. Dahle AJ.,McCollister,FP.,(1986) Hearing and otologic disorders in children with Down syndrome. Am JMental Deficiency. 90 (6):636-642.
  15. Lewis MP, Bradford Bell E, Evans AK. (2011) A comparison of tympanometry with 226 Hz and 1000 Hz probe tones in children with Down syndrome. Int J PediatrOtorhinolaryngol.75:1492-5.
  16. NICE (2008)National Institute for Health and Clinical Excellence Clinical guideline 60: Surgical Management of Otitis Media with effusion in Children. February 2008. Available from: Accessed 30.12.2016
  17. Pappas DG, Flexer C, Shackelford L. (1994) Otological and habilitative management of children with Down syndrome. Laryngoscope. 104:1065-70
  18. Shott SR, Joseph A, Heithaus D. (2001) Hearing loss in children with Down syndrome.Int J Pediatr Otorhinolaryngol . 61:199-205.
  19. Sheehan PZ, Hans PS. (2006) UK and Ireland experience of bone anchored hearing aids (BAHA) in individuals with Down syndrome.Int J PediatrOtorhinolaryngol.70:981-6.
  20. Paulson LM, Weaver TS, Macarthur CJ. Outcomes of tympanostomy tube placement in children with Down syndrome--a retrospective review. Int J PediatrOtorhinolaryngol. 2014 Feb;78(2):223-6.
  21. Doğru, M, Kuran, G, Haytoğlu, S, Dengiz, R. and Arıkan, OK, 2015. Role of laryngopharyngeal reflux in the pathogenesis of otitis media with effusion.Int Adv Otol,11, pp.66-71.
  22. McDermott AL, Williams J, Kuo MJ, Reid AP, Proops DW. (2008) The role of bone anchored hearing aids in children with Down syndrome. Int J PediatrOtorhinolaryngol. 72:751-7.
  23. Hans PS, England R, Prowse S, Young E, Sheehan PZ. (2010) UK and Ireland experience of cochlear implants in children with Down syndrome. Int J PediatrOtorhinolaryngol. 74:260-4.
  24. NICE Guidelines Otitis media Revised 2011 30.12.16

Authors:

Miss Emma McNeill, Consultant ENT Surgeon

Sunderland Royal Hospital, City Hospitals Sunderland

Dr Shiela Puri, Consultant Community Paediatrician

Leeds Community Healthcare NHS Trust

Acknowledgements

Sunshine and smiles, Parent support group for children with Down syndrome, Leeds

Dr WaheedaPagarkar , Consultant AudiovestibularPhyscian, University College Hospitals LondonDrAnuradhaShetye, Consultant AudiovestibularPhyscian, Stevenage

BAAP British Association of Audiovestibular Physicians

British Association of Paediatricians in Audiology

(BAPO – British Association of Paediatric Otolaryngologists)

Lynn Silvester, Specialist Speech and language therapist Clinical Lead for Deaf and hearing impaired.Leeds Community Healthcare NHS Trust

Teachers for the hearing impaired, DAHIT team, Leeds City Council

DSMIG UK Steering group members (2014-2016)

28.5.2017